Contents

Search


osteochondrodysplasia

Inherited developmental disorders causing dwarfism & abnormal body proportions. Management: 1) no definitive therapy available 2) symptomatic treatment directed at secondary features a) degenerative arthritis b) corrective surgery for cleft palate c) monitor for development of cataracts d) laser therapy to prevent retinal detachment 3) patient education a) avoid contact sports b) counseling for psychological problems associated with short stature

Related

dyschondroplasia Ellis-van Creveld syndrome (chondroectodermal dysplasia) fucosidosis mucosulfatidosis; multiple sulfatase deficiency; Austin variant of metachromatic leukodystrophy Schwartz-Jampel syndrome (SJS1)

Specific

achondrogenesis achondroplasia acromesomelic chondrodysplasia Cantu syndrome; hypertrichotic osteochondrodysplasia chondrodysplasia Blomstrand type (BOCD) chondrodysplasia punctata Desbuquois dysplasia fibrochondrogenesis Jansen-type metaphyseal chondrodysplasia metatropic dwarfism type 2 (Kniest dysplasia, Kniest syndrome) multiple epiphyseal dysplasia (EDM) pseudoachondroplasia pycnodysostosis Schmid type metaphyseal chondrodysplasia; includes: spondylometaphyseal dysplasia Japanese type spondyloepiphyseal dysplasia Stickler syndrome or hereditary progressive arthroophthalmopathy X-linked recessive chondrodysplasia punctata 1

General

developmental bone disorder osteochondropathy (disorder of bone & cartilage)

References

Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2191-92